Successful Aflibercept Treatment for Choroidal Neovascularization in a Rare Case of Optic Disc Melanocytoma

Objective: Rare disease Background: Optic disc melanocytoma (magnocellular nevus of the optic disc) is a benign congenital pigmented tumor, usually unilateral, asymptomatic, and mostly discovered in routine examinations in adult patients; however, it is associated with choroidal neovascularization. It can be difficult to differentiate it from juxta-papillary choroidal melanoma. Aflibercept is a monoclonal antibody targeting vascular endothelial growth factor A (anti-VEGF-A). This report describes a 54-year-old man with a diagnosis of optic disc melanocytoma complicated by choroidal neovascularization who was treated with intravitreal injection of anti-VEGF-A with intermittent follow-up. Case Report: A 54-year-old man received a diagnosis of asymptomatic right optic disc melanocytoma in a routine examination in 2015. We decided to follow up every 6 months, and 1 year later, due to growth of the lesion, we performed magnetic resonance imaging to exclude malignant transformation. The patient was lost to follow-up from 2019 to 2021. He came back 6 years after diagnosis, and fundus examination revealed multiple perilesional and macular exudates. We diagnosed a neovascular membrane by multimodal imaging. According to recommendation from our expert colleagues at the Curie Institute, he was treated with intravitreal injection of anti-VEGF-A with intermittent follow-up, and the evolution was favorable. Conclusions: Optic disc melanocytoma is a rare benign tumor, but neovascularization can occur in <1% of cases. Because it is a very pigmented lesion, we need multimodal imaging to diagnose choroidal neovascularization. Choroidal neovascularization associated with magnocellular nevus of the optic disc can be successfully treated by intravitreal VEGF-A in a "treat and extend" protocol.